A father has spoken out about the devastating and incredibly rare genetic disease that has 'stolen' his son from him.

Steve Givin’s son Shea, 8, thrived until the age of six when his health very suddenly and without warning deteriorated. Within a week the previously cheeky, bright, golf-loving boy had lost the ability to put his clothes on correctly or spell his name.

Just months later - and within a matter of days - he was unable to see, speak, move or feed himself.He had developed the incredibly rare, genetic brain disorder called Adrenoleukodystrophy (ALD).

Steve Givin¿s son Shea, 8, (pictured before he became ill) thrived until the age of six when very suddenly and without warning his health deteriorated

‘It was September 2010 when we took the kids on a caravan holiday to Anglesey,’ said Steve, 33, who is also father to five-year-old twins Malachy and Maebh.

‘His mother Nicki and I noticed Shea suddenly change – he started bumping into furniture, putting his clothes on backwards and not being able to put lids on his colouring pens.

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‘His behaviour also went downhill very quickly – at the time we thought it was attention seeking behaviour because he was jealous of the twins.’

The following week Shea returned to school and Steve and Nicky, 31, organised a chat with his teacher on his first day back.

They wanted to warn staff that Shea was going through a naughty patch and that they suspected sibling jealousy was to blame.

After Shea's trip to Lapland his health suddenly deteriorated and within days he was unable to speak, see, feed or move. His mother Nicki (pictured with husband Steve and the twins) is now his full-time carer. It is not known whether Shea will live another two months of ten years

But by the end of the day they received a phone call from the school.

‘His teacher told me that he clearly wasn’t the boy they had sent off on holidays six weeks previously – he couldn’t even write his name,' said Steve, a construction worker who is involved in the build of the new Alder Hey Hospital. ‘They advised us to take him to the doctors.’

Shea and his parents visited their local GP who tested his co-ordination by asking him to carry out simple tasks and when he saw how difficult he found it to put a lid on a pen he immediately ordered tests.

It was advised Shea go to Alder Hey Hospital in Liverpool, a hospital that specialises in the care of sick children.

'Shea loved golf and I thought it might cheer him up to to take him to the golf range - he loved it there,' said Steve.

'But it was like giving a club to
one-year-old. He used to be great at the game - by the age of three he
was already hitting balls 150 yards away.'

When Shea's appointment arrived, doctors ordered a CAT scan which revealed two abnormalities in his brain.

Before Shea became ill he was 'cheeky', bright and loved to play golf with his father. By the age of three, the young Everton fan was able to hit a golf ball more than 150 yards. Within the space of a week of developing the condition, Shea was unable to write his name, put his clothes on and his behaviour deteriorated drastically

WHAT IS ADRENOLEUKODYSTROPHY (ALD)?

ALD is a rare, genetic disorder that is characterised by the breakdown of the myelin sheath - that surrounds the nerve cells in the brain - and the gradual deterioration of the adrenal glands.

It is thought to affect one in 50,000 Britons.

Patients accumulate high levels of saturated, very long chain fatty acids in the brain and adrenal cortex because they do not produce enzymes that break down fatty acids

Onset of the classic – and most serious - childhood form of ALD only affects boys and tends to occur between the age of 4 and 10.

Initial symptoms include behavioural changes such as withdrawal or aggression, poor memory, and poor school performance.

There is no cure and treatment usually involves medication to improve symptoms, a no-fat diet and daily doses of Lorenzo's Oil.

Lorenzo's oil was created by a father whose son, Lorenzio, suffered with ALD.

His oil was high in oleic acid and was found to lower the fatty acids in the brain.

While there has been scepticism surrounding how much it can improve symptoms, experts in the field do admit that the oil works more effectively than any other medical approach that had been tried.

At first they thought it was cancer but they soon realised that they couldn't be tumours because they were too high up in Shea's brain.

'When we heard that we were relieved - for me and Nicky the worst thing it could be was cancer,' remembered Steve.

On September 18th, Shea was admitted for further investigation.

The following day he was given a CAT scan which finally revealed what was wrong with him.

'I
was almost relieved when I was told that they'd worked out what was wrong with him.

My first question was how they could fix it. But then they told me they couldn't.

'The doctor then proceeded to tell Nicki and I what to expect and it was like being hit by a train.

As they looked over to a healthy-looking Shea is his hospital bed, Steve and Nicki discovered he would go blind within five
months, lose the ability speak or move and eventually become a
vegetable.

Adrenoleukodystrophy (ALD) is a rare,
genetic disorder that was only discovered as a disease in its own right
1993.

Only women can carry the defective gene but only boys can develop the
variant Shea suffers with. Mother Nikki did not know she was a carrier.

It is thought to affect one in 50,000 people although some experts believe it effects less than that.

Richard Appleton, a consultant
Paediatric Neurologist at Alder Hey, said: 'ALD is certainly one of the
rarest diseases affecting
children in the UK. I have diagnosed only five children with this
disorder in the 23 years I have worked with children with neurological
diseases at Alder Hey Children’s Hospital.'

The condition is characterised by the breakdown of the myelin sheath that surrounds the nerve cells in the brain.

The sheath is responsible for messages being transmitted and efficiently along the nerve cells.

The condition also triggers the
progressive dysfunction of the adrenal gland, a gland responsible for a
wide array of bodily functions including secreting steroid hormones such
as cortisone and testosterone.

In the months that followed Shea's diagnosis (pictured left with mother Nicki, father Steve and younger five-year-old twin siblings Maebh [right] and Malachi [left]), the family visited Legoland, Lapland, Centerparks and Eurodisney to create happy memories

Those with ALD accumulate high levels of saturated, very long chain fatty acids in the brain and adrenal cortex because they do not produce the enzyme that breaks down fatty acids in a normal manner.

'It's hard because Shea doesn't show any
emotions, we don't know if he can hear and he can't see.

When I get home from
work I rub my nose on him so he knows its me.

I hope he knows its me.'

There are several forms of ALD. Onset of the classic – and most serious - childhood form that only affects boys tends to occur between the age of 4 and 10.

Initial symptoms include behavioural changes such as withdrawal or aggression, poor memory, and poor school performance.

There is no cure for the condition
and treatment usually involves medication to help improve symptoms as
well as a no-fat diet and daily doses of Lorenzo's Oil, named for
Lorenzo Odone whose story is chronicled in the 1993 movie Lorenzo's Oil

Within a matter of months Shea was blind, could not speak, eat or move. he had developed adenoleukodystrophy, a very rare, genetic disorder that affect the body's nervous system.

Lorenzo's oil was created by the father whose son, Lorenzio, suffered with ALD. His oil was high in oleic acid and was found to lower the fatty acids in the brain.

While there has been scepticism surrounding the treatment, many
experts in the field do admit that the oil works more effectively than
any other medical approach currently available.

Several days later, on the Friday,
Shea was discharged and Steve said they felt overwhelmed when they
returned home and Shea asked if he could go out to play with friends.

'He just seemed so normal and well. But he was really sick,' he said.

The family decided to make the most of the little time they had with their son as he was.They visited Eurodisney, Legoland and then went to Lapland.

Steve said that his younger children Malachi (left) and Maebh (centre) still spend a great deal of time with their brother Shea (pictured before he became ill). Steve said that they do remember what he used to be like as he would often playfight with them

But on Shea's return from the Christmas resort, Shea's health deteriorated rapidly.

He contracted septic tonsillitis and, because his immune system was so weak, he lost his sight, speech and ability to move or eat within days.

Since then his health has remained largely the same although he has developed dystonia (muscle spasms) that cause him to arch his back and twitch.

Shea, who spends his days with Nicki,
who is his full time carer, is on an array of drugs to improve his
immune system, control his sleep cycle because he cannot see light or
dark as well as sedatives to calm him when he is unwell.

'It's hard to interact as he doesn't
show any emotions. He just get upset if he's in pain. But when I get
home from work I rub my nose on him so he knows its me. I hope he knows
its me,' added Steve.

Shea's poor health means he is routinely taken to hospital for treatment for minor infections that quickly escalate.

Malachi (top right) has tested negative for the gene that causes the degenerative disease. Maebh (bottom right) may be a carrier - which means she could pass it on to her sons - but Steve and Nicki want to wait until she is older to be tested

Steve admitted that life is very hard due to Shea's condition 'We give him all the love in the world and wouldn't be without him, but it's hard. We've considered having more children but it's just too much work with Shea's illness.

'I'd give my life to have my boy back.'

Despite Shea's condition, Maebh and Malachi still spend a lot of time with their big brother but they are all too aware of the the sibling they have lost.

'Shea used to play fight with them all the time when he was well - they remember the boy he was and ask if he will ever get better,' said Steve.

Since Shea's diagnosis, Steve's son Malachy has undergone a genetic test to discover he is at risk of developing ALD. Several weeks ago, Shea’s family were given good news: Malachy's results came back negative.

Malachy's sister, Maebh, is yet to be tested, and there is a 50/50 chance that she will be a carrier, like her mother, but it is extremely unlikely that she will develop the disease.

Steve and Nicki have decided not to get Maebh tested yet, as she is highly unlikely to have the disease and they want to wait until she is older and can decide for herself if she wants to know if she is a carrier.

Shea continues to have treatment for his symptoms at Alder Hey Hospital.